Characteristics of the study population
| Total subjects N=570 | |
|---|---|
| Study follow-up time (years), median (IQR) | 5.5 (3.8–5.7) |
| No./patient over study period, median (IQR) | |
| iPExs | 0 (0–2) |
| oPExs | 4 (2–7) |
| Rate of oPEx/patient/year, median (IQR) | 0.9 (0.5–1.5) |
| Minimum time (days) between | |
| Consecutive oPEx, median (IQR) | 150 (63–324) |
| oPEx and iPEx, median (IQR) | 56 (21–184) |
| Female, n (%) | 266 (46.7) |
| Age at diagnosis (years), n (%) | |
| ≤2 | 359 (63.0) |
| >2 | 211 (37.0) |
| Age*, median (IQR) | 21 (11.5–32.9) |
| CFTR functional class, n (%) | |
| I–III | 463 (81.2) |
| IV–V | 77 (13.5) |
| Missing | 30 (5.3) |
| Pancreatic insufficient, n (%) | 441 (77.4) |
| CFRD*, n (%) | 71 (12.5) |
| FEV1% predicted*, median (IQR) | 72% (51.9%–89.2%) |
| BMI Z-score, mean (SD) | −0.1 (1.1) |
| Positive culture†, n (%) | |
| Stenotrophomonas maltophilia | 27 (4.8) |
| Burkholderia cepacia complex | 54 (9.6) |
| Staphylococcus aureus | 147 (26.2) |
| Haemophilus influenzae | 12 (2.14) |
| Aspergillus species | 97 (17.3) |
| MRSA | 9 (1.6) |
| Achromobacter xylosoxidans | 9 (1.6) |
| Pseudomonas aeruginosa | |
| Never | 151 (26.9) |
| Intermittent | 302 (53.7) |
| Chronic | 109 (19.4) |
*At each subject's first observation in the study period.
†Based on a positive culture or P. aeruginosa culture status (never, intermittent, chronic, as per Leed's criteria) in the 12 months prior to entry into the study cohort; eight patients were missing microbiology data.
BMI, body mass index; CFRD, cystic fibrosis-related diabetes; CFTR, CF transmembrane conductance regulator; FEV1% pred, FEV1 % predicted; MRSA, methicillin-resistant S. aureus.